Supratentorial white matter blurring associated with voltage-gated potassium channel-complex limbic encephalitis

Neuroradiology. 2015 Dec;57(12):1203-9. doi: 10.1007/s00234-015-1581-x. Epub 2015 Aug 21.

Abstract

Introduction: Limbic encephalitis (LE) associated with voltage-gated potassium channel-complex antibodies (VGKC-LE) is frequently non-paraneoplastic and associated with marked improvement following corticosteroid therapy. Mesial temporal lobe abnormalities are present in around 80 % of patients. If associated or preceded by faciobrachial dystonic seizures, basal ganglia signal changes may occur. In some patients, blurring of the supratentorial white matter on T2-weighted images (SWMB) may be seen. The purpose of this study was to evaluate the incidence of SWMB and whether it is specific for VGKC-LE.

Methods: Two experienced neuroradiologists independently evaluated signal abnormalities on FLAIR MRI in 79 patients with LE while unaware on the antibody type.

Results: SWMB was independently assessed as present in 10 of 36 (28 %) compared to 2 (5 %) of 43 non-VGKC patients (p = 0.009). It was not related to the presence of LGI1 or CASPR2 proteins of VGKC antibodies. MRI showed increased temporomesial FLAIR signal in 22 (61 %) VGKC compared to 14 (33 %) non-VGKC patients (p = 0.013), and extratemporomesial structures were affected in one VGKC (3 %) compared to 11 (26 %) non-VGKC patients (p = 0.005).

Conclusion: SWMB is a newly described MRI sign rather specific for VGKC-LE.

Keywords: Epilepsy; Limbic encephalitis; MRI; VGKC; White matter.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Autoantibodies / immunology
  • Cerebrum / immunology
  • Cerebrum / pathology*
  • Diffusion Tensor Imaging / methods*
  • Female
  • Humans
  • Limbic Encephalitis / immunology*
  • Limbic Encephalitis / pathology*
  • Male
  • Middle Aged
  • Potassium Channels, Voltage-Gated / immunology*
  • Reproducibility of Results
  • Sensitivity and Specificity
  • White Matter / immunology
  • White Matter / pathology*
  • Young Adult

Substances

  • Autoantibodies
  • Potassium Channels, Voltage-Gated